PURPOSE: To analyze audiologic findings in children with oculo-auriculo-vertebral spectrum (OAVS). This was a multicenter study performed by 2 universities, the Universidade Federal de Ciências da Saúde de Porto Alegre (UFCSPA) and the Universidade Federal do Rio Grande do Sul (UFRGS). The patients presented at the Genetic Clinic at UFCSPA, and only patients with clinically suspected OAVS were included. All patients who agreed to be part of the study were then referred to the Hearing Clinic at UFRGS, where they underwent hearing evaluations. RESULTS: Five children, aged between 2 years and 12 years were included (2 girls and 3 boys). The subjects underwent 2 examinations to evaluate their Brainstem Auditory Evoked Potential (BAEP) and otoacoustic emissions (OAE). The BAEP evaluation findings were as follows: Right ear (RE) was 20 dB; Left Ear(LE), 60 dB; RE, 30 dB, LE, 60 dB; RE, 65 dB; LE, 20 dB; RE, 35 dB; LE, 20 dB; RE, 20 dB; LE, 15 dB. In 2 children, it was not possible to evaluate OAE because both of their REs were malformed. All the evaluated patients had interpeak latency with normal scores. The ears with conductive hearing loss showed a delay in the absolute latencies and missing OAE. CONCLUSION: An electrophysiological hearing evaluation was necessary to confirm the audiologic diagnosis and to improve the audiologic findings relating to OAVS, all of which will facilitate more rapid intervention for possible hearing alterations.